Danilo M. Valle Cruz, M.D. | 01.12.

16 | SURGERY

PEDIATRIC SURGERY II: Respiratory and Gastrointestinal Conditions

o

BLACK TEXTS = Texts from the presentation / lecture;

ITALICIZED TEXTS = Transcripts from the recording / Lecturers inputs;
GREEN TEXTS = Texts / Info from the old file/previous transes;
BLUE TEXTS = Input from the reference book
RED TEXTS = Emphasized content

o
o

The Book-Reference for this lecture is NOT Schwartz Principles

of Surgery but Fundamentals of Pediatric Surgery by Peter
Mattei, 2011 Part V VIII.

LECTURE OUTLINE
I. RESPIRATORY CONDITIONS
1. Congenital Diaphragmatic Hernia (CDH)
2. Diaphramatic Eventeration
3. Congenital Pulmonary Airway Malformation (CPAM)
4. Congenital Lobar Emphysema (CLE)
5. Pulmonary Sequestration
II. GASTROINTESTINAL CONDITIONS
1. Esophageal Atresia (EA) and Tracheoesophageal
Fistula (TEF)
2. Gastrointestinal Reflex (GER)
3. Gastrointestinal Atresia/Stenosis
a. Pyloric Atresia
b. Pyloric Stenosis
4. Neonatal Intestinal Obstruction
5. Duodenal Malformations
a. Duodenal Atresia
b. Duodenal Stenosis
c. Duodenal Web
6. Jejuno-Ileal Atresia
7. Colonic Atresia and Stenosis
a. Colon Atresia
b. Colon Stenosis
8. Malrotation and Midgut Volvulus (MGV)
9. Meconium Ileus
10. Necrotizing Enterocolitis

RESPIRATORY CONDITIONS
1. CONGENITAL DIAPHRAGMATIC HERNIA (CDH)
EPIDEMIOLOGY
o 1:4000-5000 live births
o 1:2000 stillbirths included
o Syndromic Variants
Have been linked to chromosomal defects
such as Trisomy 18, 19, 21-23; 15q 24-26
deletions, Turners syndrome
o Associated anomalies: 40%, associated with >50
syndromes
PATHOPHYSIOLOGY
o During the formation of the diaphragm, the pleural
and the coelomic cavities remain in continuity by
means of the pleuroperitoneal canal.

The posterolateral communication is the last to be

closed by the developing diaphragm.
Failure of w/c leaves a posterolateral defect
allowing the viscera to fill the chest cavity.
Pleuroperitoneal membranes separate the pleural
and abdominal cavities by the 8th week of
gestation.
There are two main types based on the location of
the defect: Bochdalek Hernia and Morgagni
Hernia

A. BOCHDALEK HERNIA
Posterolateral defect
Occurs in 80-90%, L>R
With a (+) sac in <20%
Bilateral affectation is rare
Grossly, there is no defect, but there is a sac that
covers the defect

Clinical Manifestations of Bochdalek Hernia

o Respiratory distress
Factors contributory to respiratory distress:
Mediastinal shifting from air-filled bowel
Pulmonary hypertension
Pulmonary hypoplasia
o (+) Bowel sounds at the chest cavity
o Scaphoid abdomen
Diagnosis of Bochdalek Hernia
o Prenatal Ultrasound done at 11-24 weeks (at an
average of 15 weeks)
o Chest X-Ray +/- Contrast Study is diagnostic
Right-sided lesion: lobar compression
o Echocardiography
To assess the degree of pulmonary
hypertension
To identify coexisting cardiac anomaly

B. MORGAGNI HERNIA
Anterior defect
Occurs in <2% of all CDH
Failure of the crural and sternal portions of the
diaphragm to fuse
Can occur in either side at the junction of the
septum transversum and the thoracic wall
Unilateral, right-sided in 90%
Bilateral affectation in 7%
Typically, a hernia sac is present

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 1 of 12

Herniated viscera (OILS = omentum, intestines,

liver, spleen)
Malrotation may also be found

Clinical Manifestations of Morgagni hernia

o Asymptomatic in majority of cases
o Rarely diagnosed in the newborn period
o If
symptomatic,
manifestations
include
generalized epigastric discomfort and vomiting or
coughing due to intermittent obstruction
o May be acute in presentation
o Intestinal ischemia with necrosis and perforation
o Gastric volvulus may be present
o Tamponade in pericardial herniation
Diagnosis of Morgagni hernia
o Chest X-Ray (AP): well-defined air-fluid levels in
the midline
o Chest X-Ray (Lat): retrosternal space herniation
o Contrast Study or CT Scan: confirmatory

DIFFERENTIAL DIAGNOSES
o Congenital Pulmonary Airway Malformation
o Pulmonary Sequestration
o Bronchopulmonary Cysts
o Neurogenic Cysts
o Cystic Teratomas
TREATMENT
o Surgical Repair Patch
o Transabdominal
o Open VS Laparoscopic Approach
o Reverse the pulmonary hypertension
o Extracorporeal Membrane Oxygenation (ECMO)
PROGNOSIS
o 50-80% survival

2. DIAPHRAMATIC EVENTRATION
PATHOPHYSIOLOGY
o Abnormal elevation of the diaphragm resulting in
a paradoxical motion during respiration
Normally, upon inspiration, the diaphragm
contracts downwards to increase negative
intrathoracic
pressure;
in
paradoxical
respiration, diaphragm fails to contract
downwards
o Interferes with proper mechanics and function of
both lungs
o Eventrated diaphragm rises on inspiration,
causing the mediastinum to shift and compress
the contralateral lung
o Which diaphragm is lower?

When do we say that it is diaphragmatic

eventration? When the left side is more than 1
rib level HIGHER than the right or the right side
is more than 2 rib levels HIGHER than the left.
TYPES
o Congenital
Increased development of the central tendon
or muscular portion
Unilateral (L>R) > Bilateral
May be indistinguishable from a hernia sac in
congenital diaphragmatic hernia
Uncommonly can result to pulmonary
hypoplasia
Persistent fetal circulation (PFC) and
pulmonary hypertension are not usually seen
o Acquired
Due to paralysis of the phrenic nerve
Mediastinal tumors
Congenital heart surgery as in BlalockTaussig surgery for patients with
Tetralogy of Fallot
Birth trauma as in Erbs palsy during
difficult delivery
Why birth trauma? REVIEW your
anatomy of the nerve supply of the
diaphragm.
The
phrenic
nerve
originates mainly from the 4th cervical
nerve, but also receives contributions
from the 4th and 3rd cervical nerves
(C3-C5). Brachial plexus injuries can
cause paralysis to various regions in
the arm, forearm, and hand depending
on the severed nerves.
CLINICAL MANIFESTATIONS
o May be asymptomatic
o Typically presents acutely as respiratory distress
and tachypnea in the newborn
o Feeding intolerance from discoordinated sucking
and breathing
o Recurrent respiratory infection and wheezing
o Exercise intolerance in older children
DIAGNOSIS
o Chest X-Ray to note the rib levels of the
diaphragm
o Motion Studies as in Ultrasound or Fluoroscopy
confirmatory
TREATMENT
o Small Eventration may be observed

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 2 of 12

Large Eventration plication; sometimes we

excise the central portion of the diaphragm for
biopsy to differentiate it from CDH
Transthoracic or Transabdominal
Open Approach or Minimally Invasive
Techniques

3. CONGENITAL PULMONARY AIRWAY

MALFORMATION (CPAM)
Previously known as congenital cystic adenomatoid
malformation (CCAM) and congenital lung cyst
PATHOPHYSIOLOGY
o Cystic proliferation of the terminal bronchiolar
structures with an associated suppression of
alveolar growth
o Cysts are lined by cuboidal or columnar epithelium
o May occur in any lobe or simultaneously in both
lobes
o Site: common at the left lower lobe
o Type I may be confused with congenital
diaphragmatic hernia (CDH), congenital lobar
emphysema, pneumatocoeles, and pulmonary
sequestration
CLASSIFICATION
o Stocker (Pathologic) Classification most
important classification is type I (because this type
has a favorable prognosis)
I single, large, or multiple, >2cms
II smaller, more numerous, <2cms
III resembles fetal lung (solid) without
microscopic cysts
o Anatomic Classification (currently used)
Macrocystic - >5mm (echolucent)
Microcystic - <5mm (echogenic)
May occur in any lobe or simultaneously in
both lobes
CLINICAL MANIFESTATIONS
o From none at all to severe respiratory failure
o Repeated infection in older infants and children
DIAGNOSIS
o Chest X-Ray multiple lucencies in the thoracic
cavity
o Ultrasound and CT Scan may be definitive; CT
is more sensitive
TREATMENT
o Complete Lobectomy
Urgent especially if with severe respiratory
distress
Approach: Transthoracic
Be cautious in the diagnosis (rule out CDH)

PROGNOSIS
o Macrocystic (I) favorable
o Microcystic (III) unfavorable
COMPLICATIONS
o Malignant
degeneration
lipoblastoma

to

pulmonary

4. CONGENITAL LOBAR EMPHYSEMA (CLE)

PATHOPHYSIOLOGY
o Progressive hyper-expansion of 1 lung lobes
o Lung inflates but cannot deflate (almost the same
as emphysema seen in adults)
o It causes atelectasis of the adjacent lobe and
mediastinal shift
o It can be secondary to intrinsic bronchial
obstruction from poor bronchial cartilage
development or extrinsic compression of the
ipsilateral bronchus
o Manifests during the first few months of life
o Life-threatening in the newborn period
o Older infants present with less respiratory distress
o Differential:
Pneumothorax,
Treatment
is
Thoracostomy by Needle or Tube
o Site: common in the upper lobe (L>R) followed by
the right middle lobe, may also occur in the lower
lobes
o Associated
abnormalities
include
cardiac
anomalies:
Cardiac defects occur in as much as 14%
Enlarged left atrium or major vessel
compressing the ipsilateral bronchus
CLINICAL MANIFESTATIONS
o Mild respiratory distress to full-fledged respiratory
failure
o Hyperexpanded hemithorax is pathognomonic
DIAGNOSIS
o Chest X-Ray usually sufficient
Hyperlucency
with
adjacent
lobar
compression and atelectasis
o CT Scan for stable patients, confirmatory
o Bronchoscopy contraindicated because the lung
is already hyperexpanded; doing bronchoscopy
will further hyperexpand the lung and cause
rupture resulting to pneumothorax
DIFFERENTIAL DIAGNOSES
o Foreign Body
o Mucus Plug
TREATMENT
o Complete Lobectomy
o Open or Thorascopic Approach
o Timing is symptom-dependent

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 3 of 12

PROGNOSIS
o Excellent

5. PULMONARY SEQUESTRATION
PATHOPHYSIOLOGY
o Cystic mass of nonfunctioning lung parenchyma
which does not communicate with the
tracheobronchial tract by normally related bronchi
o May be due to failure of the pulmonary artery to
develop fast enough to supply the whole of the
growing lung, leading to formation/persistence of
a systemic arterial supply from the aorta
TYPES
o Intralobar
90%
Left lower lobe > Right lobes
o Extralobar
10%
Variable but usually basilar and above the left
hemidiaphragm
Commonly found in cases of congenital
diaphragmatic hernia
Therefore during the repair of CDH, you may
opt to resect this lesion
CLINICAL MANIFESTATIONS
o Intralobar recurrent pulmonary infections
o Extralobar rare
DIAGNOSIS
o Chest X-Ray
o CT Scan or MRI confirmatory
o Aortography/Angiography
o Color Doppler Ultrasound presence of an
anomalous blood supply from the aorta
This would detect blood flow to the lesion
TREATMENT
o Intralobar
Segmentectomy or Lobectomy
Open or Thoracoscopic Approach
o Extralobar
May be observed
PROGNOSIS
o Excellent
REVIEW:
1. Common Differentials for CDH: CCAM, CLE, PS
2. Management for Respiratory Conditions:
a. CDH: Medica
b. CCAM: Surgical (Lobectomy)
c. PS: Surgical if Intralobar (Lobectomy)
d. CLE: Surgical (Lobectomy)

GASTROINTESTINAL CONDITIONS
1. ESOPHAGEAL ATRESIA (EA) AND
TRACHEOESOPHAGEAL FISTULA (TEF)
EPIDEMIOLOGY
o 1:4000 live births
PATHOPHYSIOLOGY
o Uncertain
o Esophagus and trachea normally divide into
separate tubes by 34-36 days of gestation
o Interruption in the separation process is the
traditional explanation to the development of TEF,
but does not explain why EA forms
ASSOCIATED ANOMALIES
o Isolated EA no definitive genetic mutation
o Syndromic EA mutations have been identified
(e.g. N-myc, Sox2, CHD7)
o VACTERL Association
Vertebral Abnormalities
: 15%
Anointestinal Abnormalities : 8%
Cardiac Abnormalities
: 38%
Tracheoesophagel Abnormalities
Renal Abnormalities
: 15%
Limb Abnormalities
: 19%
Hydrocephalus
Other Abnormalities
: 16%
o Associated Anomalies
Cardiac
: 23%
Musculoskeletal
: 18%
Anointestinal
: 16%
Anorectal Malformation : 9%
Duodenal Atresia
: 5%
Malrotation
: 4%
Genitourinary
: 15%
Head and Neck
: 10%
Mediastinal
: 8%
Chromosomal
: 6%
Pulmonary
: 2%
GROSS-VOGT CLASSIFICATION
Type A EA/Pure EA
8-10%
Type B EA + Proximal TEF
1%
Type C EA + Distal TEF (commonest)
85%
Type D EA + Proximal and Distal TEF
2%
Type E H-Type/ Isolated TEF
8%
CLINICAL MANIFESTATIONS
o Regurgitation or drooling (excessive salivation)
o Choking or coughing
o Abdominal distention (Types CDE)
o Scaphoid abdomen in pure TEF (Type A)

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 4 of 12

Presence of pneumonia (aspiration pneumonia or

chemical peritonitis Type CD)
o Associated anomalies 55% (VACTERL-H)
DIAGNOSIS
o Prenatal Ultrasound
Polyhydramnios (>90%) more than 2L
Failure to visualize a fetal stomach
Upper
esophageal
distention
during
swallowing pouch sign
o Thoracic MRI
100% sensitive, not cost-effective
Its use is still being evaluated
o Postnatal Diagnosis
Inability to pass an NGT (French 10) into the
stomach, usually stops at 12 cm level from the
nostrils or 9-10 cm from the alveolar ridge
Misdiagnosis:
coiling,
traumatic
perforation, descent via proximal and
distal fistulous tract
Plain Abdominal X-Ray: may visualize the
dilated upper pouch
Chest X-Ray: NGT coiling (French 5)
o VACTERL Work-Up
2D Echocardiography
Lateralize the arch of the aorta
Right-sided arch in 5%
Left
(approach
is
posterolateral
thoracostomy)
Cardiovascular assessment identifies
the most important predictors of survival
Spinal X-Ray/Ultrasound
Skeletal X-Ray (Babygram)
KUB Ultrasound
Bronchoscopy if stable
TREATMENT
o Medical attention to the respiratory status
Treat pneumonia with broad spectrum
antibiotics
Treat acid reflux with acid blockers or PPIs
Suctioning may be performed intermittently
o Surgical
Primary Repair rarely a surgical emergency
Delayed or Staged Repair
Long Gap EA: >3.5cms or 3 vertebral
bodies
Extremely/Ultra-Long Gap EA: >5cms
Open vs Thoracoscopic Approach
o Feeding Gastrostomy
o Ligation of the TEF in very unstable cases

To prevent reflux and to oxygenate the

involved lung
o Livaditis Circular Myotomy 1.5 cms apart, can
lengthen by about 1 cm
o Esophageal Replacement long gap; may use the
colon, stomach, jejunum, ileum
o Isolated TEF Type E ligated via the right low
cervical approach
COMPLICATIONS
o Anastomotic Leak (10-15%)
o Stricture (10-20%)
o Gastroesophageal Reflux
o Recurrence
MODIFIED WATERSON CRITERIA (SPITZ)
o Prognosis is based on weight and associated
anomalies
Group
Birth Characteristics
Survival
I
>1,500 g
97%
II
<1,500 g
59%
OR major cardiac anomaly
III
<1,500 g
22%
AND major cardiac anomaly
o Overall survival rate
Stable: >90%
Unstable: 40-60%
o PRIORITY: treat the concomitant pneumonia then
just do surgery later
2. GASTROINTESTINAL REFLEX (GER)
EPIDEMIOLOGY
o GER occurs to some degree in children
PATHOPHYSIOLOGY
o Interferes with nutrition: failure to thrive
o Damages the esophagus causing lesions:
bleeding/stricture
o Leads to pulmonary problems: reactive airway
disease, aspiration pneumonia, apnea
DIAGNOSIS
o Barium Swallow or Upper GI Series: rule out
anatomic obstruction
What is upper GI? Above the ligament of
Treitz (LOT)
UGIS: Esophagoduodenography (EGD)
May involve fluoroscopy
o 24-Hr pH Probe Study: most accurate but poorly
tolerated
o Esophageal Manometry
o Radioisotope Milk Scan: gastric emptying
o Endoscopy + Biopsy: rule in esophagitis, Barretts
esophagus

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 5 of 12

TREATMENT
o Conservative
Thickened feeding
Prone, head-up position; angle of the head is
usually 30o
o Medical
Unresponsive to conservative management
Older children with severe gastroesophageal
reflux
Gastric acid reduction:
H2 Blockers: cimetidine, ranitidine
PPI: omeprazole (most effective)
Prokinetics:
Metoclopramide
Erythromycin
Cisapride (but the problem is that it has an
arrhythmogenic side effect, especially in
adults with heart problems)
o Surgery
If medical treatment fails
If there is an anatomic abnormality (e.g. large
hiatal hernia as in patients with Morgagni
hernia, stricture)
Open vs Laparoscopic Approach
Antireflux Procedures
If the medical management fails
If there is recurrent pneumonia due to
aspiration of gastric contents
Several types of antireflux techniques:
Nissen Fundoplication: complete wrap
(360o) of the esophagus using the
stomach
Thal Fundoplication: anterior partial wrap
(270o)
Additional Gastrostomy: done if the
patient is <1 year old and has a (+)
neuromotor swallowing disorder as in
neurologically-impaired patients (e.g.
those with cerebral palsy)
Other Surgical Procedures
Nissen-Rossetti Modification: preserves
the short gastric vessels
Hill Posterior Gastropexy
Boerema Gastropexy
Boix-Ochoa Procedure
Belsey mark Operation
Toupet Posterior Fundoplication

3. GASTROINTESTINAL ATRESIA/STENOSIS
EPIDEMIOLOGY
o 1:2000 live births
o 1/3 of the neonatal surgical admission
PATHOPHYSIOLOGY
o Morphological discontinuity
May occur as Atresia or Stenosis
a. PYLORIC ATRESIA
EPIDEMIOLOGY
o 1:100,000 live births
o <1% of all GIT atresia
CLINICAL MANIFESTATIONS
o Slight upper abdominal distention
o Non-bilious emesis as the obstruction occurs only
at the pyloric area, hence no reflux of bile into the
stomach
DIAGNOSIS
o Plain Abdominal X-Ray
Single bubble +/- air-fluid levels
With no air beyond the stomach
o Abdominal Ultrasound
Dilated pyloric canal
TYPES
I Mucosal atresia
58%
II Separated by fibrous cord
34%
III Two ends of the stomach and the duodenum 8%
are separated by a short gap
TREATMENT
o Type I web excision via gastrostomy +
pylorotomy
o Type II and III gastroduodenostomy (bypass
procedure)
ASSOCIATED ANOMALIES
o 30-40%
o Epidermolysis Bullosa most common
o Aplasia Cutis Congenita
o Multiple Intestinal Atresia
During laparotomy, inspect other parts of the
GIT to note for this associated anmaly
b. PYLORIC STENOSIS
PATHOPHYSIOLOGY
o Most common surgical cause of vomiting in
infancy
o Hypertrophy of the circular musculature
surrounding the pylorus (gastric outlet obstruction,
G.O.O.)
ETIOLOGY
o Unknown (genetic and environment)
o Factors implicated:

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 6 of 12

Hormonal, abnormal pyloric innervation,

extracellular matrix proteins, smooth muscle
cells, growth factors
Environmental: Maternal anxiety, drug use,
feeding practices, oral erythromycicn in
neonates
EPIDEMIOLOGY
o 1:300 live births ,1.5-4/1000 live births
o Usually at 4-6 weeks of age because it takes some
time for the muscles of the pylorus to hypertrophy
o Male (2-5:1), 1st born child
o Primarily seen in full term infants but may
also be seen in preterm (3.1%) which poses a
difficult diagnostic challenge (as the preterm
feeds/vomits less)
o Tends to run in families
(+) family history (parent)
15x sibling
5-20% of sons and 2.5-7% of daughters
DIAGNOSIS
o History
Projectile emesis as if it is hitting the wall
10-30 mins after feeding. with 2-3 oz (60-90
mL ~normal gastric capacity) feeding volume
Non-bilious
Coffee ground (10%) gastritis/esophagitis
o Physical Examination
Abdominal distention
Visible reverse gastric peristaltic wave
Olive-shaped mass in the mid-epigastrium or
RUQ
Difficult to feel but can be identified in 7090%
Palpate during feeding; flex the
extremities towards the abdomen to
prevent contraction of the abdominal
muscles
o Laboratory
Electrolytes (low Na/Cl/K) due to vomiting
ABGs
Metabolic imbalance:
Hypokalemic
Hypochloremic
Metabolic
Alkalosis
(vomiting causes acid losses)
Bilirubin (Increased B1/Unconjugated)
<5% of cases
Transient deficit in glucuronyl transferase
activity
Normalizes with hydration and feeding
resumption

Urinalysis - Paradoxic aciduria (as the body

tries to compensate)
o Radiology
Plain Abdominal X-Ray large dilated
stomach, no distal air (gastric outlet
obstruction, G.O.O.)
Ultrasound 95% diagnostic., operator
dependent
Muscle Thickness
=/> 4 mm
Channel Length
=/> 16 mm
Diameter
=/> 14 mm
Negative or Equivocal: do UGIS
o Upper Gastrointestinal Series (UGIS)
Gold standard
String Sign: single streak of barium in the
lumen of elongated pylorus
Beak Sign: beginning the elongated pyloric
channel
Double Track Sign: double streaks of Ba
passing through the narrow pylorus
TREATMENT
o Never a surgical emergency
o Resuscitation
Correct fluid and electrolyte imbalance and
acid-base deficit x 24-48 hrs. preoperatively
with D5 0.45 NaCl + K at 2-4 mEq/kg
150-175 mL/kg/24hrs
Urine output >1mL/kg/hr
o The compensation for severe metabolic alkalosis
is respiratory acidosis which is produced by
hypoventilation.
o Patient with severe metabolic alkalosis therefore
will not breathe well post-operatively.
o Fredet-Ramstedt Pyloromyotomy via Laparotomy
or Laparoscopy
o Feeding: between 6-18 hrs. post-operatively
o Emesis encountered in 65-90%
COMPLICATIONS
o Perforation
o Bleeding
o Wound infection
o Recurrence
MEDICAL MANAGEMENT
o Reserved for patients with poor operative risks
o Requires prolonged post-pyloric feeding via
feeding tube (6-12 months)

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 7 of 12

4. NEONATAL INTESTINAL OBSTRUCTION

CLINICAL MANIFESTATIONS
o Cardinal Signs:
History of polyhydramnios (>2000 ml)
Bilious vomiting in the newborn means
obstruction unless proven otherwise
Can be seen also in patients w/ sepsis &
adynamic ileus
Physical
examination:
+/abdominal
distention depends on the level of GIT
involved
Failure to pass meconium w/in the 1st 24-48
hours of life
LOCATION
o Proximal
Bilious vomiting
Minimal abdominal distention
Plain Abdominal X-Ray: paucity of gas, no
distention; rule out malrotation and midgut
volvulus (MGV)
o Distal
Bilious vomiting
Abdominal distention
Plain
Abdominal
X-Ray:
calcification
(meconium
peritonitis
secondary
to
perforation
of
the
small
intestine),
pneumatosis/PPN (necrotizing enterocolitis)
DIAGNOSIS
o Plain Abdominal X-Ray
Complete obstruction with/no distal air in the
pre-sacral area
Incomplete obstruction with some gas more
distally in the bowel
o Contrast Enema Study water-soluble and not
barium enema as barium enema can result to
peritonitis
(+) Microcolon
Jejunoileal Atresia
Meconium Ileus (Small Intestine)
(-) Microcolon
Hirschsprungs Disease
Small Left Colon Syndrome (seen in
children of mothers who are diabetic)
Meconium Plug (Large Intestine)
5. DUODENAL MALFORMATIONS
Intrinsic Obstruction
o Duodenal Atresia complete
o Duodenal Stenosis/Web/Duplications partial

Extrinsic/Partial Obstruction
o Annular Pancreas
o Malrotation with Ladds Bands
o Preduodenal Portal Vein
Other Associated Biliary Abnormalities
o Biliary Atresia (BA)
o Gallbladder Agenesis
o Common Bile Duct (CBD) Stenosis
o Choledochal Cysts (CDC)
ASSOCIATED ABNORMALITIES (50%)
Trisomy (21-30%)
Isolated Cardiac Defect (30%)
Other GIT Anomalies (25%)
o Prematurity (45%)
o Growth Retardation (33%) may be due to
deprivation of swallowed amniotic fluid
CLASSIFICATION
o Stenosis mostly involve the 3rd or 4th part of the
duodenum
o Atresia
LEVEL OF OBSTRUCTION
o Preampullary: 10-15% (non-bilious vomiting)
o Postampullary: 85% (bilious vomiting)

a. DUODENAL ATRESIA
GREY-SKANDALAKIS CLASSIFICATION
o Type 1: intact diaphragm or membrane that is
formed by mucosa and submucosa; muscularis is
intact (windsock deformity; inserting an NG tube
will cause intussusception)
o Type 2: two blind ends are connected by a short
fibrous cord with intact mesentery
o Type 3: blind ends are separated and with
mesenteric defect
DIAGNOSIS
o 30-59% (to 81%) have history of maternal
polyhydramnios in the third trimester
o Down Syndrome is present in approximately 1/3 of
all cases
o Prenatal Ultrasound
dilated fluid-filled stomach and duodenum
usually detected between 7th and 8th month
age of gestation or even as early as 5th month
age of gestation
double bubble sign 44% of cases if with (+)
history of maternal polyhydramnios
o Decompression by Vomiting or Orogastric Tube
(OGT) Aspiration
Instill 40-60 mL of air to reproduce the double
bubble

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 8 of 12

Plain Abdominal X-Ray

Usually sufficient to confirm the diagnosis if
there is antenatal suspicion of duodenal
obstruction and the clinical presentation is
consistent with proximal bowel obstruction
Double bubble sign; air-filled stomach and
1st portion of the duodenum with no distal air
If with distal air, the air from the stomach
inserts into the bifurcation
CLINICAL MANIFESTATIONS
o Bilious vomiting in the first few hours of life due to
incompetence of the pylorus
o Non-bilious thereafter
o On physical examination, +/- abdominal
distention, abdomen is usually scaphoid
o OGT aspirate: >20 mL suggests obstruction,
<5mL is normal
MANAGEMENT
o OGT decompression
o Resuscitation with fluid and electrolyte correction
o Semi-Elective Surgery
Complete metabolic profile
CBC, coagulation studies
Ultrasound abdominal and spinal
2D Echocardiography
o Emergency Surgery
If malrotation with midgut volvulus cannot be
excluded
o Surgery depends on the type of atresia and the
associated abdominal conditions:
Annular Pancreas (36%)
Malrotation (30%)
APV (4%)
2nd Distal Web (3%)
Biliary Atresia (2%)
o Duodeno-duodenostomy
Tapering Duodenoplasty
(Open vs Laparoscopic Approach)
Side-to-Side
Diamond-Shaped
Proximal transverse to digital longitudinal
Preferred
COMPLICATIONS
o Late post repair (12-15%)
Megaduodenum
Intestinal Motility Disorder
Gastroesophageal Reflux
PROGNOSIS
o >90% survival
o Most death are related to severe cardiac defects

b. DUODENAL STENOSIS
DIAGNOSIS
o Plain Abdominal X-Ray: double bubble sign is
usually not present
o Upper Gastrointestinal Series
TREATMENT
o Duodeno-duodenostomy
c. DUODENAL WEB
DIAGNOSIS
o Plain Abdominal X-Ray: incidental finding
TREATMENT
o Vertical Duodenotomy
o Partial Excision of Web
Avoid injury to the inserting pancreatico-biliary
duct
Oversewing the mucosa
Horizontal closure
6. JEJUNO-ILEAL ATRESIA
EPIDEMIOLOGY
o Incidence: 1:2,000-5,000 live births (M=F)
PATHOPHYSIOLOGY
o Believed to be due to antenatal fetal mesenteric
vascular accident:
In Utero Volvulus
Malrotation
Gastroschisis
Intussusception
Internal hernia
CLINICAL MANIFESTATIONS
o Bilious vomiting
o Progressive abdominal distention
LOUW CLASSIFICATION
Type I
Mucosal atresioa with intact muscularis
Type II
Atretic ends connected by a fibrous band
Type III A
Atretic ends are separated by a V-shaped
mesenteric defect
Type III B
Apple peel or Christmas tree deformity
Type IV
Multiple, string of sausage or string of
beads appearance
DIAGNOSIS
o Plain Abdominal X-Ray
Level
Calcification (12%)
Note: the more distal the obstruction, the more
distended the abdomen, the greater the
number of obstructed loops
o Barium Enema
Rotation and its caliber

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 9 of 12

Small intestine versus large intestine

If the diagnosis is uncertain or distal
obstruction is suspected
(+) Microcolon
Jejunoileal Atresia
Meconium Ileus
(-) Microcolon
Hirschsprungs Disease
Small Left Colon Syndrome
Meconium Plug
TREATMENT
o Laparotomy
Once the diagnosis of complete intestinal
obstruction is considered (i.e. staggered airfluid levels, clinical picture)
o Surgery depends on the type of atresia
Resection + Anastomosis (End to Back)
Ileostomy + Distal Mucus Fistula (DMF) if
ischemic or necrotic

7. COLONIC ATRESIA AND STENOSIS

EPIDEMIOLOGY
o Incidence: 1:15,000 to 20,000 live births
PATHOPHYSIOLOGY
o In utero vascular compromise of the mesentery to
the large bowel
o Most affected areas are the transverse and the
sigmoid colon which have floppy mesentery that
are prone to volvulus
a. COLON ATRESIA
CLINICAL MANIFESTATIONS
o Failure to pass meconium within the 1st 24 hours
of life
o Abdominal distention
o Bilious vomiting
DIAGNOSIS
o Plain Abdominal X-Ray
Dilated intestines with air-fluid levels
Soap bubble appearance (air + meconium)
o Contrast Enema
Blind distal end of a microcolon
TREATMENT
o Right colon and transverse colon: primary
resection with end-to-end anastomosis or
colostomy
o Sigmoid: colostomy with subsequent closure after
3-6 months
PROGNOSIS
o 90-100% survival

8. MALROTATION AND MIDGUT VOLVULUS (MGV)

EMBRYOLOGY
o 6th week midgut herniates into the umbilical cord
o 10th week begins its return to the abdominal
cavity
o Midgut undergoes 270o counterclockwise rotation
around the superior mesenteric artery which
results in the ligament of Treitz being in the LUQ
and the cecum in the RLQ
PATHOPHYSIOLOGY
o MGV can occur at any age but commonly in the
first few weeks of life
o May lead to infarction of the entire midgut
(necrosis can occur in 1-2 hours)
o May result to short gut syndrome
o Occurs in 70% of infants with malrotation
CLINICAL MANIFESTATIONS
o Feeding intolerance
o Bilious emesis (77-100%) 1st sign of MGV
o Abdominal pain/ irritability
o Hematochezia/ hematemesis
o Circulatory collapse
o Early Stage: minimal signs
o Advanced: erythema and edema of the anterior
abdominal wall, shock
o Chronic Obstructive Symptoms
Intermittent abdominal pain
Vomiting (occasionally bilious)
Failure to thrive
May be misdiagnosed as GERD
DIAGNOSIS
o Plain Abdominal X-Ray
Non-specific, neither R/I or R/O malrotation
Paucity of gas throughout the intestine with
few scattered AFL
Double bubble sign (20%)
Duodenal Triangle triangular gas shadow
in the RUQ (upright view)
o Upper Gastrointestinal Series
Gold standard, most reliable diagnostic tool
Incomplete rotation with duodenojejunal
junction, displaced to the right
Failure of the ligament of Treitz or
duodenojejunal flexure to cross to the left of
the midline
Suggestive of MGV
Cork-screw appearance with right-sided
bowel loops
Cut Off Sign
Beak Sign

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 10 of 12

Barium Enema: displaced or high lying cecum,

unreliable
o Ultrasound and CT Scan: suboptimal
TREATMENT
o Exploratory Laparotomy
o Detorsion in a Counter-Clockwise Direction
o Ladds Procedure Appendectomy
The procedure involves surgical division of
Ladd's bands, widening of the small intestine's
mesentery, performing an appendectomy and
correctional placement of the cecum and
colon.
Advanced Ischemia
o Reduce the volvulus without Ladds Procedure
then 2nd look operation after 24-36 hours
o Conservative resection
Recurrent Volvulus
o Uncommon
o Can still occure after Ladds Procedure
MORTALITY
o 4+-8%

9. MECONIUM ILEUS
EPIDEMIOLOGY
o Affected patients almost always have cystic
fibrosis
PATHOPHYSIOLOGY
o Produces obstruction from impaction of
meconium in the distal ileum
o Inspissation is caused by lack of pancreatic
enzymes
CLINICAL MANIFESTATIONS
o Bilious vomiting
o Progressive abdominal distention
o Failure to pass meconium
DIAGNOSIS
o Prenatal Ultrasound
Intra-abdominal or scrotal calcifications
Distended bowel loops
o Plain Abdominal X-Ray
Dilated bowel loops with no air-fluid levels
Ground glass appearance small bubbles of
gas entrapped in the inspissated meconium
Eggshell
pattern

intraperitoneal
calcification suggestive of prenatal intestinal
perforation
o Contrast Enema under Fluoroscopy
Confirmatory
Microcolon
Pellets of meconium in the terminal ileum

Failure to reflux into the terminal ileum

(suggestive of complicated meconium ileus or
an associated atresia)
TREATMENT
o Uncomplicated
Water-soluble contrast enema
Commonly with the use of gastrograffin
Every 12 hours for several days
o Complicated
Perforation
Vascular compromise
Resection of distended terminal ileum
Flushing of meconium pellets with diluted Nacetylcystein or NSS
End to End Anastomosis
Ileostomy
Ileostomy with Distal Mucus Fistula (DMF)
Bishop-Koop Anastomosis
Mikulicz Double Barrel Enterostomy

10. NECROTIZING ENTEROCOLITIS

EPIDEMIOLOGY
o Most frequent and lethal gastrointestinal disorder
affecting almost exclusively the stressed
premature neonate (1-3:1000 live births)
o Multifactorial in origin
PATHOPHYSIOLOGY
o Mechanism is still underfined
o Breakdown of mucosal barrier and colonization
with indigenous bacterial flora
o The more immature the infant at birth, the greater
the risk of acquiring NEC
o Usually develops at the 10th day of life (7-21 days
of life) when the gastrointestinal tract is colonized
by coliforms
o Common isolates include: Escherichia coli,
Enterobacter spp., Klebsiella spp., coagulasenegative Staphylococcus
RISK FACTORS
o Prematurity
o Initiation of enteral feeding
o Bacterial infection
o Intestinal ischemia (Asphyxia)
o Umbilical artery cannulation
o Patent ductus arteriosus
o Cyanotic heart disease
o Maternal cocaine abuse
o Premature and Enteral Alimentation
The only consistent epidemiology precursors
of NEC

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 11 of 12

 No enteral feeding in 10%

Full Term Infants
Low risk
5-25% of cases develops within the first few
days of life
STAGES (BELL CLASSFICATION)
o I suspect, show features of NEC
o II definite, established case
o III advanced, with evidence of necrosis or
perforation
o Modification includes clinical, gastrointestinal, and
radiographic findings
CLINICAL MANIFESTATIONS
o Milk feeding intolerance 1st sign
o Physiologic instability
o Abdominal distention (ileus)
o Hematochezia
o Bilious vomiting
o Anterior abdominal wall edema and erythema
o Ascites
o Jaundice
DIAGNOSIS
o Hematologic Profile
o Imaging Studies
Plain Abdominal X-Ray
Ileus
Pneumatosis intestinalis (pathognomonic,
98%)
Portal venous gas (10%)
Intraperitoneal fluid (10%)
Persistently dilated bowel loops
Pneumoperitoneum foot ball sign in
supine view
Ultrasound: portal venous gas, pneumatosis,
ascites
MRI prohibitively difficult in preterm infants,
more feasible in full term but rarely necessary
Contrast Study - relatively contraindicated
TREATMENT
o Conservative Management 75% success in
uncomplicated cases
NPO for 10-14 days
OGT with intermittent suction
Broad-spectrum antibiotics
Hypotension crystalloids, blood products,
and pressors or inotropes
Mechanical
ventilation
to
maintain
oxygenation if needed
Total parenteral nutrition
o Surgical Indications

Pneumoperitoneum
Hepatoportal vein gas
Significant acidosis (pH<7.2) after volume
resuscitation
Bacteria/intestinal contents by paracentesis
(equivocal cases)
No significant clinical improvement after
several days
o Surgical Approach
Laparotomy
Resection with ostomy or end-to-end
anastomosis, then 2nd look operation
after 24-48 hours if there is massive
involvement
Peritoneal Drainage
To relieve tension PPN
To establish a controlled fistula
1/3 survive without requiring additional
surgery
Convert to laparotomy if no improvement
after 48-72 hours
Both approaches have similar outcome
COMPLICATIONS (due to diagnostic delay)
o Perforation
o Sepsis
o Profound metabolic acidosis
o Disseminated intravascular coagulation
o Respiratory failure
o Cardiovascular collapse
o Death
o Strictures
Develop in 20% of cases (medically or
surgically treated)
Contrast enema is mandatory before intestinal
continuity is established
Ileostomy closure (between 2 and 2.5 kgs)
OUTCOME
o I 85% survival
o II 65% survival
o III 35% survival

PI: Arellano | Gagui | Galvan | Pamintuan | Timbang

SURGERY | PEDIATRIC SURGERY: Respiratory and Gastrointestinal Conditions
PROPERTY OF AUFSOM Batch 2017
V3.2 s2015-2016

Page 12 of 12